Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis

Molecular Genetics and Metabolism

Volumn 94, Issue 2, 2008, Pages 222-233

  Sleat, David E ^a,b   El Banna, Mukarram ^a   Sohar, Istvan ^a   Kim, Kwi Hye ^a   Dobrenis, Kostantin ^c   Walkley, Steven U ^c   Lobel, Peter ^a,b  

^a RUTGERS UNIVERSITY   (United States)

^b RUTGERS ROBERT WOOD JOHNSON MEDICAL SCHOOL   (United States)

^c New York State Office of Mental Retardation and Developmental Disabilities   (United States)

Author keywords

Hypomorph; Lysosomal storage disease; Mouse model; Neuronal ceroid lipofuscinosis

Indexed keywords

LYSOSOMAL PROTEASE TRIPEPTIDYLPEPTIDASE 1; PEPTIDASE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CONTROLLED STUDY; DISEASE COURSE; DISEASE SEVERITY; ENZYME ACTIVITY; FEMALE; LIFESPAN; MOUSE; NEURONAL CEROID LIPOFUSCINOSIS; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION;

ANIMALS; BRAIN; DISEASE MODELS, ANIMAL; ENDOPEPTIDASES; GENE TARGETING; GENE THERAPY; LIVER; LYSOSOMES; MICE; MICE, TRANSGENIC; MITOCHONDRIAL PROTON-TRANSLOCATING ATPASES; NEURONAL CEROID-LIPOFUSCINOSES; SPECIES SPECIFICITY;

MUS;

EID: 43249097209     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2008.01.014     Document Type: Article

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